Clinical trials in childhood steroid sensitive nephrotic syndrome: the PREDNOS studies

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1 Clinical Research Facility Central Manchester University Hospitals NHS Foundation Trust Clinical trials in childhood steroid sensitive nephrotic syndrome: the PREDNOS studies Professor Nick Webb DM FRCP FRCPCH Royal Manchester Children s Hospital, Manchester UK NIHR Manchester Clinical Research Facility Nephrology for General Paediatricians Meeting, Southampton, July 2017 Supported by

2 Overview Steroid sensitive nephrotic syndrome Overview PREDNOS study PREDNOS 2 study

3 Childhood idiopathic nephrotic syndrome Incidence 2-3/100,000 children in UK Modal age of presentation 2-3y Twice as common in boys 4-6 x increased incidence in UK S. Asian population Acute onset of heavy proteinuria with subsequent development of nephrotic syndrome

4 Childhood idiopathic nephrotic syndrome Diagnosis based on clinical presentation, low plasma albumin and (very) heavy proteinuria Renal function generally normal Transient increase in plasma creatinine in small proportion secondary to hypovolaemia 25% have microscopic haematuria Blood pressure generally normal 10-20% have transient hypertension secondary to hypovolaemia

5 Initial management Large majority will have minimal change disease which will be steroid responsive Treat most with empiric course of steroids without biopsy Indications for biopsy: Age <12m or >12y Atypical presentation or tests (low C3, low egfr) Failure to respond to course of steroids

6 ISKDC regimen Prednisone (Prednisolone) 60mg/m 2 (max 80mg) daily for 4 weeks followed by 40mg/m 2 (max 60mg) on alternate days for 4 weeks Has been the gold standard regimen against which all others have been compared

7 Treatment of the presenting episode

8 PREDNOS 225 children with newly presenting SSNS 237 participants recruited by 7 th October 2014 ISKDC regimen Total 8 weeks steroids Total recruitment time 38 months Then placebo to 16 weeks Prednisolone 60mg/m 2 /d 4w Then alternate days 60mg/m 2 2w 50mg/m 2 2w 40mg/m 2 2w 30mg/m 2 2w 20mg/m 2 2w 10mg/m 2 2w Total 16 weeks steroids Results available Sept 2017

9 First patient randomised 2 nd August 2011 Dr Shankar and team, City General Hospital, Stoke on Trent

10 Participating sites Total of 124 study sites Largest paediatric study in NIHR portfolio

11 Recent studies

12 Corticosteroid therapy for nephrotic syndrome in children: 2015 update

13 Clinical course following first episode of SSNS 10-20% have no further relapses 30% have infrequent relapses 50% develop frequently relapsing* or steroid dependent + disease requiring alternative therapies * >4 relapses per year + 2 relapses on or within 14 days of stopping AD steroids

14 ISKDC relapse treatment Prednis(ol)one 60mg/m 2 until urinary remission (3 days zero or trace proteinuria) then 40mg/m 2 alternate days for 28 days Infrequent relapses treated with repeated courses Multiple relapses increase risk of steroid related adverse events

15 Steroid sparing treatments the prevention of disease relapses Alternate day prednisolone Levamisole Alkylating agents cyclophosphamide and chlorambucil Calcineurin inhibitors ciclosporin and tacrolimus Purine inhibitors mycophenolate mofetil Anti- B cell therapies rituximab

16 Evolution of PREDNOS 2 - URTI and relapses Around 50% of relapses are preceded by an URTI If URTI develops, around 50% chance of a relapse developing It seems logical that URTI is pivotal and attempts to ameliorate the URTI driven process are appropriate

17 Pre-emptive treatment of relapses Gulati et al. Clin J Am Soc Nephrol 2011; 6: children - FRNS on AD prednisolone (32 on levamisole) At time of development of URTI randomised to 7 days of daily prednisolone at same dose Remained on AD prednisolone URTI defined as Fever >38, rhinorrhoea, cough, diarrhoea Incidence of URTI-related relapse reduced Relapse rate reduced by 0.7/y (95%CI : p<0.01)

18 Unanswered questions Utility of this approach in Western nations Different pattern of URTI less fever, diarrhoea etc. Utility in children receiving other therapies ciclosporin, tacrolimus, MMF, cyclophosphamide, rituximab +/- AD prednisolone? Cost-effectiveness Adverse-effect risk? Particularly effect on behaviour

19 PREDNOS 2: Primary study objective To determine whether a six day course of oral prednisolone given at the time of URTI reduces the incidence of first URTI-related relapse

20 PREDNOS UK children with FRNS ( 2 relapses in past 12m) On any long-term immunosuppressive regimen Randomised to prednisolone or placebo arm at study entry When URTI develops, parents commence child on 6 days of daily prednisolone (15mg/m2) or placebo Repeated with each URTI that develops over 12 month follow-up period

21 PREDNOS 2 URTI defined as the presence of at least 2 of the following for at least 24 hours: sore throat ear pain/discharge runny nose cough (dry/barking) hoarse voice fever >37 O C (measured using tympanometric electronic thermometer)

22 PREDNOS 2 Primary end-point development of URTI-related relapse Secondary end points relapse rate, cumulative prednisolone dose, adverse events (particularly behavioural), escalation / de-escalation of background immunosuppressive therapy Quality of Life Assessment Formal Health Economic Analysis (Frew, Birmingham) DNA sampling

23 Recruitment New sites welcome to join study First participant recruited Total 285 participants recruited 116 sites set-up 64 have recruited participants Further sites currently in set-up

24 Funding extension Rate of recruitment slower than anticipated Number of participants completing study without URTI greater than anticipated Event rate (URTI-related relapse following first URTI) lower than anticipated Further funding to increase number of participants to 360 by April 2018 We need the on-going help of Wessex centres to achieve this

25 Corticosteroid therapy for nephrotic syndrome in children: 2015 update

26 PREDNOS 2 NEEDS

27 Conclusions The PREDNOS studies have illustrated Large scale multicentre paediatric studies in District Hospitals are entirely deliverable in the UK Great willingness of DGH paediatricians to participate PREDNOS studies are answering clinically important questions Results likely to influence future UK (and international) practice Translation into changes in patient care could take place over a very short time period

28 Acknowledgements NIHR HTA for funding the studies NIHR MCRN and CRN University of Birmingham Clinical Trials Unit Birmingham Children s Hospital Clinical Trials Pharmacy All Principal Investigators and teams Children who have participated and their families

29 Thank you

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